New-onset refractory status epilepticus and its subcategory febrile infection-related epilepsy syndrome are rare devastating clinical presentations in those without pre-existing relevant history, often in schoolchildren or young adults, without a clear cause on initial investigations. A cause is later identified in up to half of adults, but in many fewer children. Patients often require protracted intensive care and are at significant risk of dying. Functional disability is common and subsequent chronic epilepsy is the norm, but some people do have good outcomes, even after prolonged status epilepticus. Patients need prompt investigations and treatment. Anaesthetic and antiseizure medications are supplemented by other treatment modalities, including the ketogenic diet. Despite limited evidence, it is appropriate to try to modify the presumed underlying pathogenesis with immune modulation early, with a more recent focus on using interleukin inhibitors. Optimising management will require concerted multicentre international efforts.

中文翻译：

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新发难治性癫痫持续状态 (NORSE)


新发难治性癫痫持续状态及其亚类发热性感染相关性癫痫综合征是罕见的破坏性临床表现，对于那些没有预先存在相关病史的人来说，通常是在学童或年轻人中，并且在初步调查中没有明确的原因。后来在多达一半的成年人中找到了病因，但在儿童中却发现了更少的原因。患者通常需要长期的重症监护，并且面临巨大的死亡风险。功能障碍很常见，随后的慢性癫痫也很常见，但有些人即使在长期癫痫持续状态后，也确实有良好的结果。患者需要及时检查和治疗。麻醉和抗癫痫药物辅以其他治疗方式，包括生酮饮食。尽管证据有限，但早期尝试通过免疫调节来改变假定的潜在发病机制是适当的，最近的重点是使用白细胞介素抑制剂。优化管理需要多中心国际协调一致的努力。